中医辨证论治肝硬化腹水70例

肝硬化系指各种原因所致的肝脏组织炎症、坏死发生的肝脏纤维化和残存肝细胞结节性再生。其病理特点为广泛的肝细胞变性和坏死,纤维组织弥漫性增生,并有再生小结节形成,早期可无症状,后期出现肝功能减退,门静脉高压和多系统受损。腹水为最常见并发症。笔者采用中医辨证论治,取得较好疗效,具体介绍如下。     

肝纤维化与抗肝纤维化

肝纤维化是组织学检查作出的一种形态学诊断,是诸多慢性肝病向肝硬化发展的必经阶段.其病理学特征为汇管区大量纤维组织增生,但并无假小叶和再生结节形成.随着肝脏病变的发展,肝细胞坏死、肝实质弥漫性结节再生、纤维组织包绕、分隔,终致肝脏结构变形,发展为不可逆转的肝硬化.能否将病变终止在肝纤维化阶段或逆转至正常,是近年颇受肝脏病     

特发性门脉高压症的临床病理学特点

目的 探讨特发性门脉高压症(idiopathic portal hypertension,IPH)的临床病理学特点.方法 回顾性分析了9例IPH的临床及病理学资料,并对其肝脏标本进行常规病理学及免疫组化研究.结果 9例IPH中,5例首发症状为上消化道出血和黑便,3例体检发现脾大脾亢而无临床症状,1例以血管瘤入院.人院检查脾肿大7例,胃底食道静脉曲张6例,腹水征4例,贫血者6例,肝功能正常或接近正常9例.病理组织学显示9例肝小叶结构基本正常.均未见假小叶形成及肝细胞坏死;9例均有不同程度汇管区纤维化,3例汇管区纤细的不全纤维间隔形成并向肝实质延伸,6例有门脉末支管壁纤维化;9例中有6例小叶内肝细胞有不同程度的水肿变性,5例肝窦有不同程度的扩张,2例肝窦扩张较明显,肝细胞萎缩,呈血管瘤样结构,2例有轻-中度肝腺胞3区大泡脂变.脾脏组织学符合淤血性脾肿大病理表现.结论 IPH的临床表现与其他原因所致的肝硬化门脉高压相似,肝穿组织病理学可除外肝硬化,并有一定的特征.诊断时应与各种原因所致肝硬化门脉高压,肝窦阻塞综合征等相鉴别.     

12例肝结节状再生性增生临床分析

目的对肝结节状再生性增生（NRHL）的临床、病理及诊断分析总结，期望提高临床医师对本病的认识。方法从300例因脾功能亢进而行脾切除和肝脏活组织检查病例中选出病理诊断符合NRHL的病例12例，分析其病史、临床症状和体征、实验室检查、诊断及处理等资料，且随访治疗效果。结果12例NRHL中6例明确诊断为系统性红斑狼疮，1例克罗恩病，1例疑诊溃疡性结肠炎。应用肾上腺皮质激素治疗6例，免疫抑制剂3例。11例有门脉高压；所有患者均无病毒性肝炎史；肝功能轻度受损；病理特征为肝实质内结节状再生性增生，门脉周围轻度纤维化和汇管区散在淋巴细胞浸润，门静脉分支狭窄和闭塞，无肝坏死表现；术前均被诊断为肝硬化伴门脉高压；行手术治疗后临床症状明显缓解，随访患者多数病情稳定。结论NRHL可能与免疫和肝脏血液循环障碍有关；以门脉高压为主要表现，应与肝硬化鉴别；诊断依靠肝脏楔形活检；处理门脉高压可使临床状况得到改善。     

肝硬化的影像学特征

肝硬化是以肝细胞变性、坏死、结节样再生、纤维组织增生和肝脏结构紊乱为特征的一种病理过程。根据诊断目的 ,肝硬化的传统Ｘ线检查方法主要有 3种 :( 1)食道和胃肠钡餐造影 ,以显示门静脉高压所引起的食道下段和胃底的静脉曲张 ;( 2 )门静脉造影 ,直接显示门静脉阻塞以及侧支循环的形成和曲张 ;( 3)选择性肝动脉造影 ,以显示肝动脉、肝实质和门静脉分支等情况。超声、ＣＴ和ＭＲ能够较好显示肝硬化的肝脏形态异常和继发性改变。现分别介绍如下。一、ＣＴ(一 )肝脏大小和形态　肝硬化的纤维疤痕使肝脏缩小 ,而再生结节和脂肪浸润又使肝脏增…     

免疫机制在特发性门脉高压症发病中的作用

特发性门脉高压症临床上较少见,主要表现为脾大、贫血和门脉高压,无肝硬化和肝外门静脉及肝静脉阻塞,肝功能基本正常。肝脏组织学变化主要为汇管区和门脉周围纤维化及炎性细胞浸润。血流动力学可发现脾门脉血流量增加。此文就免疫机制在其发病中的作用做简要综述。     

免疫机制在特发性门脉高压症发病中的作用

特发性门脉高压症临床上较少见,主要表现为脾大、贫血和门脉高压,无肝硬化和肝外门静脉及肝静脉阻塞,肝功能基本正常.肝脏组织学变化主要为汇管区和门脉周围纤维化及炎性细胞浸润.血流动力学可发现脾门脉血流量增加.此文就免疫机制在其发病中的作用做简要综述.     

肝纤维化中的贮脂细胞

肝纤维化是组织学检查的一种形态学诊断,其病理学特征是汇管区纤维结缔组织增多,增多的纤维结缔组织常形成细小的条索和菲薄的间隔或由汇管区向小叶内伸延,但并无假小叶及再生结节形成,其本质是细胞外基质(ECM)合成过多。有关ECM的来源一直在争论中,曾认为它主要来源于肝细胞,但目前大多数学者认为贮脂细胞(FSC)是产生ECM的主要细胞,肝细胞、Pit细胞、内皮细胞等也参与其中。本文就FSC在肝纤维化的作用及其调控因素作一综述。     

肝纤维化几种病理染色方法及意义归纳

目的:归纳肝组织各种病理切片染色方法,探讨不同方法在大鼠肝纤维化病理诊断中的意义。方法:将实验大鼠分为正常组和模型组,每组各8只,模型组腹腔注射30%CCl4橄榄油溶液2. 0ml/kg,1次/4天,共7次,建立肝纤维化模型。取材肝组织制作病理切片,分别进行HE染色、弹力胶原纤维染色、网状纤维染色、Masson(马松)三色染色、天狼星红染色。结果:正常组大鼠肝组织各种染色均呈现正常形态。模型组大鼠肝脏切片HE染色示肝细胞变性、坏死,呈现大面积假小叶组织结构,属坏死后肝硬变;弹力胶原纤维染色示大量胶原纤维沉积,汇管区周围胶原纤维沉积,纤维条索较粗且染色着色较深,包裹,已形成假小叶;马松染色示大量蓝色胶原纤维沉积,自汇管区周围向外延伸,纤维条索较粗且染色着色较深,表明胶原纤维较多,已形成假小叶;网状纤维染色示网状纤维失去正常分布状态,自汇管区周围网状纤维塌陷、融合、增粗、包裹并已形成假小叶;天狼星红染色示各型胶原纤维自汇管区向外扩展,偏光镜下观察可见胶原沉积、扩展已形成假小叶,其中大量红色I型胶原纤维、绿色III型胶原,少量疏网状II型胶原纤维和淡黄色IV型胶原纤维。结论:HE染色可以观察细胞形态,弹力胶原纤维染色、网状纤维染色、Masson(马松)三色染色均能诊断肝纤维化,而天狼星红染色可以对肝纤维化组织中各型胶原纤维进行分型。     

21例特发性门脉高压临床及病理特点分析

目的分析特发性门脉高压(idiopathic portal hypertension,IPH)的临床及病理特点。方法回顾性分析2012年1月—2016年12月在解放军第三〇二医院住院治疗(资料完整)的21例IPH患者的临床及病理特点。结果 21例IPH患者中,男女比例6∶15,平均发病年龄(38.1±12.7)岁,临床以门脉高压症表现为主,肝功能无明显减退,主要并发症为上消化道出血及腹水。21例肝组织病理主要表现为肝细胞板排列基本正常,无假小叶形成,汇管区扩大,门静脉周围纤维化,门脉周围有不同程度的细胞浸润,血管紊乱,中央静脉及小叶间静脉扩张,肝窦扩张,窦周纤维化。结论 IPH患者门脉高压和肝功能损害不平行,门脉高压表现较重,确诊仍须病理学检查,治疗以防治并发症为主。     

Histological changes in the liver and portal hypertension subsequent to repeated intraportal injections of killed E. coli in the dog

The etiology of idiopathic portal hypertension (IPH) is not known. To obtain clues to the pathogenesis, an attempt was made to produce a hepatic lesion similar to that in IPH by repeated injections of aggregated killed non-pathogenic E. coli directly into the portal vein. In the treated dogs, histology of the liver showed dense fibrosis in the portal tract and an aberrant vasculature around the portal area after 1 month. Portal pressure was elevated and middle-to-small-sized portal branches were decreased in number as studied by portography. These changes closely mimic those seen in human IPH. The possibility is discussed that chronic entrance of an antigen such as bacteria from the intestine to the portal venous system plays an etiologic role in IPH.     

Fibulin-5 is involved in phlebosclerosis of major portal vein branches associated with elastic fiber deposition in idiopathic portal hypertension.

Aim: In cases of idiopathic portal hypertension (IPH), the deposition of elastic fibers in the major portal vein branches and peripheral portal tracts is a common and characteristic histological finding, which may be related to the disease's pathogenesis. This study aimed to clarify the mechanism of this portal fibroelastosis. Methods: The expression of fibulin-5 and fibrillin-1, proteins essential for elastogenesis, was examined in IPH livers (n = 15) using immunohistochemistry. Liver specimens obtained from patients with chronic viral hepatitis (CVH)/liver cirrhosis (LC) (n = 12) and normal/subnormal livers (n = 10) were used as controls. Results: In IPH livers, immunohistochemical labeling of fibulin-5 was observed in the major portal vein branches in eight cases (53%), and the distribution corresponded to that of elastic fibers in the vessel walls, while the peripheral portaltracts totally lacked fibulin-5 in spite of the presence of dense elastic fibers. In CVH/LC and normal livers, fibulin-5 expression was absent or faint throughout the sections. Fibrillin-1 was detected in the connective tissue of the hilar region and peripheral portal tracts in IPH, CVH/LC and normal livers, with the expression varying greatly among cases. Conclusions: These results suggest that fibulin-5, rather than fibrillin-1, expressed in the major portal vein branches of IPH livers is related to phlebosclerosis, leading to an increase in presinusoidal vascular resistance and portal hypertension. In addition, the mechanism of fibroelastosis may differ between the major portal vein branches and peripheral portal tracts of IPH livers.     

特发性门脉高压肝移植术后随访第3年再发1例报告及文献复习

目的 复习1例特发性门脉高压(IPH)患者接受肝移植(LT)后第3年出现病情“再发”,并进行了相关文献复习,以提高对该病的认识。方法 报道1例我们诊治的IPH患者的病例资料,并检索MEDLINE、EMBASE、万方等数据库经LT治疗的IPH患者的研究报道,分析其治疗和转归。结果 本文报道的病例为57岁女性,因消化道出血、腹水行LT术,组织病理学检查诊断为IPH;术后随访第3年病情复发,行经皮肝穿刺活检术,病理学检查提示结节性再生性增生(NRH)、轻度汇管区炎症及纤维化,提示IPH再发;文献检索到81例LT治疗的IPH患者,其中42例在LT前诊断为肝硬化;LT后最长随访时间为248个月,8例死亡,其中5例分别在首次LT后3.5月～14年进行肝活检,组织病理学检查提示NRH,3例分别于LT后第7月、第3年和第14年出现具有门脉高压表现的NRH。结论 具有严重的门脉高压或肝功能衰竭的IPH患者需要LT治疗。少数患者在LT后可能出现IPH“再发”。     

Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India The Japan idiopathic portal hypertension study*,+

ABSTRACT— Morphological changes of the liver were studied in 24 autopsy cases of noncirrhotic portal hypertension of unknown etiology (idiopathic portal hypertension, IPH), and in 123 surgical biopsies from such patients. For comparison, 15 whole-cut liver slices from autopsy cases of noncirrhotic portal fibrosis (NCPF) from India were also studied. Liver pathology was very similar in IPH and NCPF, characterized by phlebosclerotic changes and perivascular fibrosis of the portal vein system, and parenchymal atrophy perhaps secondary to portal circulatory insufficiency. The distribution of lesions was uneven, and despite marked fibrosis and occasional surface nodularity, there was no diffuse pseudonodule formation in the parenchyma. Surgical specimens showed similar changes except for more frequent portal cellular infiltrates, but the changes seen in one biopsy specimen were limited and not always diagnostic. It seems that IPH of Japan and NCPF of India are the same disease, and perhaps hepatoportal sclerosis elsewhere is also the same disease.     

A case of idiopathic portal hypertension after renal transplantation

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.     

A case of idiopathic portal hypertension after renal transplantation

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.     

Pathology and pathogenesis of portal venopathy in idiopathic portal hypertension: Hints from systemic sclerosis

Idiopathic portal hypertension (IPH) is a non-cirrhotic presinuosidal portal hypertension of unknown etiology. Stenosis of smaller portal veins with portal fibrosis is a pathologic hallmark of IPH. Association of systemic sclerosis (SSc) with IPH is recognized, and similar pathologic features are reported in small portal tracts and skin of IPH and SSc, respectively. In addition, levels of transforming growth factor-β (TGF-β) and connective tissue growth factor are elevated in serum and in affected skin and portal tracts of these two diseases, suggesting that IPH share fibrogenetic mechanisms with SSc. Endothelial to mesenchymal transition (EndMT) of microvasculatures of skin could be responsible for dermal fibrosis in SSc. In IPH, EndMT of portal vein endothelium via TGF-β/Smad activation may also be involved in small portal venpathy. In IPH, enhanced expression of pSmad2 in venous endothelium of smaller portal veins was associated with reduced CD34 expression. CD34 and S100A4, and CD34 and type I collagen were colocalized to portal vein endothelium in IPH. Such myofibroblastic phenotypes may be responsible for periportal-venous deposition of collagen and compressive portal venous obliteration. These small portal venous lesions may in turn lead to portal venous insufficiency followed by subcapsular atrophy in IPH.     

Portal-systemic shunting in a patient with normal portal vein pressures and histological evidence of idiopathic portal hypertension

Although idiopathic portal hypertension (IPH) is clinically characterized by portal hypertension and marked splenomegaly, we have experienced a case of spontaneous portal-systemic shunt without splenomegaly in whom the liver histology resembled IPH but with normal portal pressure. We admitted a 64 year old man who had suffered from hepatic encephalopathy for 2 years. Laparoscopy revealed a dark grey liver with a sharp edge and a concave surface. Examination of a liver biopsy specimen revealed peri-portal fibrosis consistent with IPH. A single, large, portal-systemic shunt was identified by percutaneous transhepatic portography. The shunt arose from the left gastric vein and flowed through the left renal vein into the inferior vena cava. No varices were identified. There were no morphological changes in the hepatic or portal veins. Portal vein pressure was normal. There was a slight difference between the portal pressure and the wedged hepatic vein pressure, suggesting a presinusoidal block. This case raises important questions concerning the aetiology of IPH and the relationship between portal hypertension and the development of collateral venous circulation.     

Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a review of literature

Idiopathic portal hypertension (IPH) is characterized by dense fibrosis of portal tracts and portal venous obliteration. Little is known about the etiopathogenesis of IPH. Association of various autoimmune diseases such as systemic lupus erythematosus in IPH suggests that IPH may share immunological disturbances with such autoimmune diseases. We recently experienced two autopsy cases presenting with both diffuse scleroderma and IPH. Dense fibrosis was found in both the dermis and intrahepatic portal tract of these cases. In addition, small vascular damages were commonly observed to various degrees in these fibrotic areas of both organs. The activation of fibroblasts and vascular damages mediated by various growth factors and cytokines reportedly involved in the dermis in scleroderma might have also been operative in portal tracts in these two cases of IPH. A review of literature disclosed eight overlapping cases of IPH and scleroderma (middle- to old-aged females), and scleroderma was diagnosed earlier than IPH. These findings suggest that similar pathogenetic processes are operative in the dermis as well as in the portal tracts of the liver in these cases.